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Article / Publication Details AbstractIntroduction: Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a serious life-threatening disease. Tumor localization is crucial in EAS management. This underscores the importance of evaluating imaging methods and prognostic factors to provide a clear basis for patient diagnosis and management. Objective: To investigate imaging methods and analyze the relevant prognostic factors for EAS. Methods: The retrospective study followed 64 cases of EAS diagnosed between 1992 and 2020. Clinical features, biochemical analysis, imaging studies were collected, and survival data was followed-up and analyzed. Results: Of 64 patients, 41% were female with a mean (±SD) age at diagnosis of 47±16 years. Computed tomography (CT), 18-F fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)-CT, and octreotide scintigraphy had similar sensitivity in localizing ectopic ACTH-secreting tumors. However, in cases with negative imaging on CT, both of 18F-FDG PET-CT and octreotide scintigraphy further localized 25% tumors. The combination of all three modalities failed to further increase the sensitivity. Patients with thymic tumors survived longer than those with pulmonary or pancreatic tumors (P=0.013 and 0.047, respectively). Multivariate analyses showed that hypokalemia (P=0.004) and treatment modality (P=0.048) were independent prognostic factors. The optimal serum potassium cut-off based on maximum log-rank statistics (P=0.012) was 2.90 mmol/L. Conclusion: CT is first-choice in tumor localization in EAS. CT in combination with a nuclear medicine or molecular imaging modality is necessary for further identification of an ectopic source. Serum potassium
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